title: [Molecular pathogenesis of spongiform encephalopathies].
abstract: The understanding of the pathogenesis of transmissible spongiform encephalopathies (TSE) has advanced most significantly in the last forty years, and some scientists even feel that this group of diseases now ranks among the best understood neurodegenerative conditions. On the other hand, the research on TSE has led to several unexpected and sometimes amazing findings, which have often elicited passionate controversies. Although the human forms of these diseases are rare, the epidemic proportions of the bovine spongiform encephalopathy (BSE) forces us to urgently reconsider the question of transmissibility to humans. The present review article summarizes some of the current hypotheses about the nature of the infectious agent and about the pathogenesis of the damage in the central nervous system. In particular we discuss transgenic and knockout models which, in combination with organ transplantation techniques, are proving invaluable in the elucidation of the pathogenesis of these diseases.
authors.affiliation: Departement Pathologie, Universitätsspital, Zürich, Schweiz.
authors.forename: A
authors.initials: A
authors.lastname: Aguzzi
mesh_terms: D000818:Animals; D030801:Animals, Genetically Modified; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000503:physiopathology / Q000635:transmission; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D017096:Prion Diseases / Q000503:physiopathology* / Q000635:transmission; D011328:Prions / Q000502:physiology*; D012608:Scrapie / Q000635:transmission
chemical_list: D011328:Prions
vernacular_title: Molekulare Pathogenese der spongiformen Enzephalopathien.